Alternative Description: Gemin3
Catalogue Number: IQ204
Also known as: Component of gems 3; DDX20; DEAD box protein 20; DEAD-box protein DP103; DEAD/H BOX 20 antibody; DKFZP434H052 antibody; DP103 antibody; Gemin3 antibody; SMN interacting protein antibody;
Quantity: 0.1ml (1mg/ml)
Price: £328.00Price excludes VAT and Delivery.
Clone: 12H12;
Host/Isotype: Mouse IgG1;
Immunogen: Recombinant 6His-tag COOH-terminal domain of Gemin3 (amino acids 368-548)
Myeloma/fusion partners: SP2/0;
Specificity: Human Gemin3. Detects a band of approximately 105 kDa
Purification: Protein A;
Format: Purified antibody (from supernatant) containing PBS + 0.1% sodium azide;
Applications: E; IF; IP; WB;
View Applications KeyDilutions: Optimal antibody dilution should be determined by titration
Relevance: The survival of motor neurons (SMN) gene is the disease gene of spinal muscular atrophy (SMA), a common motor neuron degenerative disease. The SMN protein is part of a complex containing several proteins, of which one, SIP1 (SMN interacting protein 1), has been characterized so far. The SMN complex is found in both the cytoplasm and in the nucleus, where it is concentrated in bodies called gems. In the cytoplasm, SMN and SIP1 interact with the Sm core proteins of spliceosomal small nuclear ribonucleoproteins (snRNPs), and they play a critical role in snRNP assembly. In the nucleus, SMN is required for pre-mRNA splicing, likely by serving in the regeneration of snRNPs. A DEAD box putative RNA helicase, named Gemin 3 which is another component of the SMN complex, has been identified. Gemin 3 interacts directly with SMN, as well as with SmB, SmD2 and SmD3. Immunolocalization studies using mAbs to Gemin 3 show that it colocalizes with SMN in gems. Gemin 3 binds SMN via its unique COOH-terminal domain, and SMN mutations found in some SMA patients strongly reduce this interaction. The presence of a DEAD box motif in Gemin 3 suggests that it may provide the catalytic activity that plays a critical role in the function of the SMN complex on RNPs.
References: (click to view full details)
A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems- Charroux et.al ( J Cell Biol,Dec 1999)
Identification of a Novel Human Immunodeficiency Virus Type 1 Integrase Interactor, Gemin2, That Facilitates Efficient Viral cDNA Synthesis In Vivo- Seiji Hamamoto et al ( J. Virology,June 2006)
Research areas: Neuroscience; signal transduction; Splicing;
Storage: Store at +4°C for one month, or in small aliquots at -20°C for longer
Health and safety: Products that contain sodium azide (a poisonous and hazardous substance) should be handled only by trained personnel
Protocols: Immunofluorescence protocol - Formaldehyde fixation; Immunofluorescence protocol - Methanol/acetone fixation; Western Blotting Protocol;
